Spinal Arteriovenous Malformation Masquerating Zoster Sine Herpete.

1.4 MB·English

by  Lee, Ji Young

Korean J Pain 2013 January; Vol. 26, No. 1: 72-75
pISSN 2005-9159 eISSN 2093-0569
http://dx.doi.org/10.3344/kjp.2013.26.1.72
| Case Report |
Spinal Arteriovenous Malformation Masquerating
Zoster Sine Herpete
Department of Anesthesiology and Pain Medicine, Ajou University Hospital, Suwon,
*Korea University Ansan Hospital, Ansan, †Jeju National University Hospital, Jeju, Korea
Ji Young Lee, MD, Se Jin Ok, MD*, Chang Keun Oh, MD,
Sun Kyung Park, MD†, Do Wan Kim, MD, and Jong Yeun Yang, MD
Zoster sine herpete (ZSH) is difficult to diagnosis during an acute period due to the absence of the
characteristic zosteriform dermatomal rash; therefore, progression to postherpetic neuralgia is more common
than typical zoster. In addition, misdiagnosis of other neuropathic pain as ZSH is common in clinical situations.
Here, we report a case of spinal arteriovenous malformation that mimics ZSH. This is a rare condition;
therefore, high clinical suspicion for a correct diagnosis and proper examination are not easy. However, early
diagnosis and definitive treatment are essential to prevent neurologic deficit and mortality. (Korean J Pain
2013; 26: 72-75)
Key Words:
arteriovenous malformation, herpes zoster, postherpetic neuralgia, zoster sine herpete.
Herpes zoster (HZ) is a clinical syndrome where topical continuing dermatomal neuropathic pain. Before diagnosis,
skin irritation, pain and paresthesia occur along the dis- it is important to differentiate from other diseases that can
tribution of the dermatome, due to varicella zoster virus cause dermatomal neuropathic pain with an accurate
entering through the sensory nerve of the skin and is dor- physical examination [3].
mant in the dorsal root ganglia or the cerebral ganglia until This case will examine spinal arteriovenous malforma-
it is reactivated when the immune system of the body be- tion (AVM), which was misdiagnosed as ZSH.
comes weak. When pain persists for 1, 3, 4, or 6 months
or more after developing a rash, it is called postherpetic CASE REPORT
neuralgia (PHN) [1].
There are rare cases of HZ which do not form vesicular A 36-year-old man with an itching and tingling sen-
eruption on the skin. Weber [2] calls this zoster sine her- sation in the left L2 dermatome area for 3 months was
pete (ZSH). Rather than early diagnosis, in most cases, it referred to our clinic for pain management. The patient did
is diagnosed as ZSH when the patient complains of chronic not have significant past medical history, and before com-
Received August 6, 2012. Revised October 19, 2012. Accepted November 8, 2012.
Correspondence to: Jong Yeun Yang, MD
Department of Anesthesiology and Pain Medicine, Ajou University Hospital, San 5, Woncheon-dong, Yeongtong-gu, Suwon 443-721, Korea
Tel: [phone], Fax: [phone], E-mail: [email protected]
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Copyright ⓒ The Korean Pain Society, 2013
Lee, et al / Spinal Arteriovenous Malformation Masquerating Zoster Sine Herpete 73
ing to the clinic, he had been treated with gabapentin 600 cion of an upper motor neuron (UMN) lesion. In the MRI,
mg 3 times a day by a dermatologist but the symptoms an intramedullary lesion 1.2 cm in size was found in the
were not improved. There were no unusual finding in the dorsal subpial surface of the T11-12 level, and this was di-
biopsy other than subcorneal pustules, hyperkeratosis and agnosed as type II AVM in the T11/12 level of the spinal
parakeratosis; therefore, he was referred to our depart- cord (Fig. 1).
ment for pain control. The character was continuous tin- A spinal angiography was performed on the patient
gling and itching, and it was aggravated when humid. He and the results revealed that the lesion was a spinal cord
was also complaining of allodynia and paresthesia, and his AVM supplied by the anterior spinal artery from the left
visual analogue scale (VAS) was 2 out of 10 (0 = no pain, T9 intercostal artery. It was also contrasted from the left
10 = worst possible pain). His motor function was intact L1 lumbar artery (Artery of Adamkiewicz) (Fig. 2).
and there was no sensory loss. His knee jerk reflex was The patient was transferred to another hospital for re-
increased (3+/3+) thus a thoraco-lumbar spine magnetic moval of AVM with cyber knife, and after the operation,
resonance imaging (T-L MRI) was performed due to suspi- the symptoms were improved.
Fig. 1. T spine MR images
show about 1.2 cm sized
ill-defined intramedullary le-
sion which has intramedull-
ary nidus and multiple flow
voids extension to the dorsal
subpial surface is noted in
T11-12 level suggesting type
II AVM in T11/12 level of
spinal cord.
Fig. 2. Spinal angiogram
shows spinal cord AVM fee-
ding from anterior spinal ar-
tery from left T9 intercostal
artery and left L1 lumbar
artery (artery of Adamkiewicz)
and nidus of T11 level con-
firming type 2 spinal arteri-
ovenous malformation, glomus
type, T11.
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74 Korean J Pain Vol. 26, No. 1, 2013
DISCUSSION which occurs during the 4th to 8th week of embryonic de-
velopment when blood vessels are differentiated into ar-
HZ is usually diagnosed by characteristic clinical sym- teries, veins, and capillaries [7]. Normally arteries and veins
ptoms including dermatomal rash. Laboratory tests are the are connected by capillaries, but there is no true capillary
Tzank smear, a serological test such as the indirect fluo- bed in AVM; thus over time, a tangle is formed in the blood
rescent antibody method, and detection through molecular vessel connecting the artery and vein. Therefore, oxy-
biology or immunology such as tissue cultures [4]. genated arterial blood is shunted and supplied directly into
A dermatomal rash is not present in ZSH so it is diffi- the venous system which can cause temporary or perma-
cult to suspect HZ, and hence, serological, immunological, nent ischemia in the surrounding tissue. AVM can develop
or molecular biological tests are not performed during the anywhere in the body and there can be various manifes-
acute phase. Only when the pain continues chronically are tations of symptoms according to the location and blood
many cases diagnosed by the characteristics of pain. flow. Usually, it develops in the limbs, neck, and face, and
However, when a patient complains of continuing dermato- it can cause pain, congestive heart failure, and hypo-
mal neuropathic pain, a detailed history taking and accu- tension from vascular congestion and so on [8].
rate physical examination should be done to differentiate Cerebral AVM can cause seizure or severe headaches,
ZSH from sciatica, collapsed intervertebral disc, spinal but mostly there are no symptoms. Spinal AVM has a prev-
stenosis, tumor, angina, renal colic, cholecystitis, costo- alence of approximately 10% for cerebral AVM and less
chondritis, intercostal neuralgia, entrapment syndrome, than 5% for an intraspinal mass. As in this case, it usually
pleurodynia, myofascial pain syndrome, and other neuro- occurs in middle-aged men in the lower thoracic or lumbar
pathic pain before diagnosis [3]. region. Typical early symptoms are dermatomal lancinating
In this case, the patient complained of tingling pain pain, weakness/paralysis, numbness, and paresthesia [9-11].
with a VAS score of 2 and accompanying itching, par- Spinal AVM are classified by 4 types according to its
esthesia, and allodynia in the left L2 dermatome for 3 form. In this case, glomus-type (type 2) with a tightly
months. During the physical examination, motor and sen- compacted group of arterial and venous vessels inside a
sory function was intact but the knee jerk reflex was in- short segment of the spinal cord, and usually, it is supplied
creased to 3+ on both sides. Hence, an UMN lesion above by one artery. Neurologic symptoms are worsened accord-
the L2 level was suspected, an AVM in the T11/12 level was ing to the increased venous pressure [12,13].
discovered in the T-L MRI. The patient received the ap- Spinal AVM is very rare with a prevalence of less than
propriate treatment with cyber knife. 1%, shows various clinical symptoms, and is difficult to dif-
The UMN is the pathway from the brain and brainstem ferentiate from other diseases such as tumors, herniated
to the anterior horn of the spinal cord. The lower motor nucleus pulposus, multiple sclerosis, meningovascular syph-
neuron (LMN) is the pathway from the anterior horn of the ilis, tuberculosis, and transverse myelitis. However sub-
spinal cord to the skeletal muscles. In this case, only the arachnoid hemorrhage or spinal cord hemorrhage due to
knee jerk reflex was hyperactive with L2 dermatomal pain AVM rupture and the subsequent acute neurologic deficit
without any other general symptoms or symptoms in the are increasing by 2-3% every year, and the mortality at
upper extremity. Therefore, a spinal cord lesion in a level first hemorrhage reaches 10-30%; therefore, an accurate
higher than the L2 level was anticipated, and a T-L MRI differential diagnosis is very important [11,14,15]. Additio-
was performed to diagnose the AVM. Other symptoms that nally, in this case, the AVM was discovered in the MRI,
suggest an UMN lesion are weakness, paralysis, spasticity, but there are many cases which cannot be detected by MRI
and muscle atrophy, but these symptoms were not ob- thus an angiography must be performed for diagnosis.
served in our patient. Only the hyperactivity of the knee In this case, spinal AVM was accurately diagnosed
jerk reflex was observed, but if the Babinski response (the through a detailed neurologic examination, and the appro-
most sensitive indicator of the UMN) had been checked, priate treatment was given. Therefore, when a patient
it would have been more helpful in making the diagnosis complains of dermatomal neuropathic pain without a rash,
[5,6]. a detailed and accurate physical examination should be
AVM is the abnormal development of blood vessels performed before diagnosing ZSH, and laboratory tests
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Lee, et al / Spinal Arteriovenous Malformation Masquerating Zoster Sine Herpete 75
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it from other diseases that cause neuropathic pain. arteriovenous fistulas: report of a case and review of the
literature. J Urol 1975; 114: 648-52.
9. Gennuso R, Zappulla RA, Strenger SW. A localized lumbar
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